Tgf-β1 Inhibits Cftr Biogenesis and Prevents Functional Rescue of ΔF508-Cftr in Primary Differentiated Human Bronchial Epithelial Cells

CFTR is an integral transmembrane glycoprotein and a cAMP-activated Cl(−) channel. Mutations in the CFTR gene lead to Cystic Fibrosis (CF)–an autosomal recessive disease with majority of the morbidity and mortality resulting from airway infection, inflammation, and fibrosis. The most common disease-...

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Bibliographic Details
Main Authors: Snodgrass, Steven M., Cihil, Kristine M., Cornuet, Pamela K., Myerburg, Michael M., Swiatecka-Urban, Agnieszka
Format: Artigo
Language:Inglês
Published: Public Library of Science 2013
Subjects:
Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3650079/
https://ncbi.nlm.nih.gov/pubmed/23671668
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0063167
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